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Disproportionate head growth retardation in cystic fibrosis
S Ghosal1, C J Taylor, M Pickering
1Department of Paediatrics, University of Sheffield.
Insights
Children with cystic fibrosis experience stunted growth at birth, with head circumference lagging behind height and weight gains despite nutritional management. This suggests potential impacts on early brain development in cystic fibrosis patients.
Area of Science:
- Pediatrics
- Genetics
- Developmental Biology
Background:
- Cystic Fibrosis (CF) affects multiple organs, including potential impacts on growth and development.
- Early nutritional management is crucial for children with CF.
- Understanding growth patterns in CF is essential for monitoring and intervention.
Purpose of the Study:
- To analyze growth parameters (length, weight, head circumference) in infants and young children with CF from birth to 4 years.
- To assess the effectiveness of early nutritional management on growth trajectories.
- To investigate potential correlations between growth patterns and CF-related physiological mechanisms.
Main Methods:
- Longitudinal study recording monthly growth data (length, weight, head circumference) in 52 children with CF.
- Calculation of Standard Deviation (SD) scores at birth and over the first four years.
- Comparison of growth trajectories for length, weight, and head circumference.
Main Results:
- Children with CF exhibited stunting at birth (SD scores: height -1.24, weight -0.72, head circumference -1.82).
- Significant improvements in length and weight SD scores were observed during the first four years.
- Head circumference showed limited improvement, stabilizing at -1 SD below the mean from 1.5 to 4 years.
Conclusions:
- Head growth in children with CF lags behind gains in height and weight, even with good early nutritional management.
- The findings suggest potential implications for early neurodevelopment in CF.
- Data may support the role of cystic fibrosis transmembrane conductance regulator (CFTR) in the choroid plexus and ependyma.
Abstract:
Monthly increments of length, weight gain, and head circumference were recorded from birth to 4 years of age in 52 children with cystic fibrosis. At birth the children showed stunting. Standard deviation (SD) scores at birth for height, weight, and head circumference were -1.24, -0.72, and -1.82 respectively. Over the first four years, length and weight SD scores showed a consistent improvement. However, there was very limited improvement in head circumference, which stabilised 1 SD below the mean from 1.5 years to 4 years. Our data suggest that head growth lags behind gain in height and weight in children with cystic fibrosis despite good nutritional management in early infancy. The data may also support the expression of cystic fibrosis transmembrane conductance regulator in choroid plexus and ependyma.